Clinical features of 23 hepatolenticular degeneration patients with liver injure
10.3760/cma.j.issn.1008-6706.2009.04.011
- VernacularTitle:以肝损害为主的肝豆状核变性23例临床特征分析
- Author:
Jingnan LI
;
Zhaoxia LI
;
Tiangao CAO
;
Yunzhen SHI
- Publication Type:Journal Article
- Keywords:
Hepatolenticular degeneration;
Signs and symptoms;
Hepatomegaly;
Diagnosticerrors;
Ceruloplasmin
- From:
Chinese Journal of Primary Medicine and Pharmacy
2009;16(4):595-596
- CountryChina
- Language:Chinese
-
Abstract:
Objective To realise the clinical features and to reduce the diagnostic errors of hepatolenticular degeneration patients with liver injure. Methods Clinical manifestations, results of las tests and admitting diagnosis of 23 hepatolenticular degeneration cases were reviewed. Results All cases showed the elevation of serum transami-nase and/or total bilirubin. 17 cases(73.9% ) had manifestation of liver malfunction while nervous system symptoms were merely found in 2 cases. The decreased ceruloplasmin and elevated 24 hour urinary cuprum were detected in 23 and 21 cases respectively. Positive K-F rings were observed in 17 of 23 patients. Misdiagnosis rate of admitting diagno-sis exceeded 43.5%. 2 cases died after treating with penicillamine. Conclusion Liver injure that is one of the com-mon clinical features results in the diagnostic errors, but the combination detection will improve the accuracy of hepa-tolenticular degeneration.
